Anti-EPM2A/Laforin antibody (ab129321)
Key features and details
- Goat polyclonal to EPM2A/Laforin
- Suitable for: IHC-P, WB
- Reacts with: Human
- Isotype: IgG
Overview
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Product name
Anti-EPM2A/Laforin antibody
See all EPM2A/Laforin primary antibodies -
Description
Goat polyclonal to EPM2A/Laforin -
Host species
Goat -
Specificity
ab129321 is expected to recognize isoform 1 (NP_005661.1) only. -
Tested applications
Suitable for: IHC-P, WBmore details -
Species reactivity
Reacts with: Human
Predicted to work with: Rabbit, Cow, Dog, Pig, Xenopus laevis, a wide range of other species -
Immunogen
Synthetic peptide corresponding to Human EPM2A/Laforin aa 131-144 (internal sequence) (Cysteine residue).
Sequence:C-EATGHTNEMKHTTD
Database link: NP_005661.1 -
Positive control
- Human brain, cerebellum and heart tissues; Human Cerebellum lysate.
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General notes
This product was previously labelled as EPM2A', 'EPM2A, isoform 9
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Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. -
Storage buffer
pH: 7.30
Preservative: 0.02% Sodium azide
Constituents: 99% Tris buffered saline, 0.5% BSA -
Concentration information loading...
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Purity
Immunogen affinity purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab129321 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application | Abreviews | Notes |
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IHC-P |
Use a concentration of 2.5 - 3.75 µg/ml. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
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WB |
Use a concentration of 0.1 - 0.3 µg/ml. Predicted molecular weight: 37 kDa.
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Notes |
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IHC-P
Use a concentration of 2.5 - 3.75 µg/ml. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol. |
WB
Use a concentration of 0.1 - 0.3 µg/ml. Predicted molecular weight: 37 kDa. |
Target
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Function
Dual specificity protein phosphatase. May be involved in the control of glycogen metabolism, particularly in monitoring for and preventing the formation of poorly branched glycogen molecules (polyglucosans). Acts as a scaffold protein to facilitate PPP1R3C/PTG ubiquitination by NHLRC1/malin. Forms a complex with NHLRC1/malin and HSP70 and this complex suppresses the cellular toxicity of misfolded proteins by promoting their degradation through the ubiquitin-proteasome system (UPS). Isoform 2, an inactive phosphatase, could function as a dominant-negative regulator for the phosphatase activity of isoform 1. -
Tissue specificity
Expressed in heart, skeletal muscle, kidney, pancreas and brain. Isoform 4 is also expressed in the placenta. -
Involvement in disease
Defects in EPM2A are a cause of progressive myoclonic epilepsy type 2 (EPM2) [MIM:254780]; also known as Lafora disease. EPM2 is an autosomal recessive and severe form of adolescent-onset progressive epilepsy. Typically, as seizures increase in frequency, cognitive function declines towards dementia, and affected individuals die usually within 10 years after onset. EPM2 occurs worldwide, but it is particularly common in the mediterranean countries of southern Europe and northern Africa, in southern India and in the Middle East. At the cellular level, it is characterized by accumulation of starch-like polyglucosans called Lafora bodies (LBs) that are most abundant in organs with the highest glucose metabolism: brain, heart, liver and skeletal muscle. Among other conditions involving polyglucosans, EPM2 is unique in that the inclusions are in neuronal dendrites but not axons and the forming polyglucosan fibrils are associated with the endoplasmic reticulum. -
Sequence similarities
Belongs to the protein-tyrosine phosphatase family.
Contains 1 CBM20 (carbohydrate binding type-20) domain.
Contains 1 tyrosine-protein phosphatase domain. -
Post-translational
modificationsPolyubiquitinated by NHLRC1/malin.
Phosphorylation on Ser-25 by AMPK affects the phosphatase activity of the enzyme and its ability to homodimerize and interact with NHLRC1, PPP1R3C or PRKAA2. -
Cellular localization
Cytoplasm; Cytoplasm. Nucleus; Endoplasmic reticulum. Cell membrane. Nucleus. Also found in the nucleus; Endoplasmic reticulum. Cell membrane. Primarily associated with polyribosomes at the endoplasmic reticulum, also found at the plasma membrane and Cytoplasm. Under glycogenolytic conditions localizes to the nucleus. - Information by UniProt
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Database links
- Entrez Gene: 484022 Dog
- Entrez Gene: 7957 Human
- Omim: 607566 Human
- SwissProt: Q1M199 Dog
- SwissProt: O95278 Human
- Unigene: 486696 Human
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Alternative names
- Epilepsy progressive myoclonus type 2 Lafora disease (laforin) antibody
- Epilepsy progressive myoclonus type 2A Lafora disease (laforin) antibody
- EPM2 antibody
see all
Images
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-EPM2A/Laforin antibody (ab129321)
ab129321 at 3.75 µg/ml staining EPM2A/Laforin in Formalin-Fixed, Paraffin-Embedded Human brain, cerebellum tissue by immunohistochemistry.
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-EPM2A/Laforin antibody (ab129321)
ab129321 at 3.75 µg/ml staining EPM2A/Laforin in Formalin-Fixed, Paraffin-Embedded Human heart tissue by immunohistochemistry.
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Anti-EPM2A/Laforin antibody (ab129321) at 0.1 µg/ml + Human Cerebellum lysate in RIPA buffer at 35 µg
Developed using the ECL technique.
Predicted band size: 37 kDa
Protocols
Datasheets and documents
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SDS download
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Datasheet download
References (0)
ab129321 has not yet been referenced specifically in any publications.